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- Title
Neuroblastoma of Undifferentiated Subtype, Prognostic Significance of Prominent Nucleolar Formation, and MYC/MYCN Protein Expression.
- Authors
Wang, Larry L.; Suganuma, Rie; Ikegaki, Naohiko; Tang, Xao; Naranjo, Arlene; McGrady, Patrick; London, Wendy B.; Hogarty, Michael D.; Gastier‐Foster, Julie M.; Look, A. Thomas; Park, Julie R.; Maris, John M.; Cohn, Susan L.; Seeger, Robert C.; Shimada, Hiroyuki
- Abstract
BACKGROUND: This study sought to investigate biological/clinicopathological characteristics of neuroblastoma, undifferentiated sub-type (NBUD). METHODS: This study examined 157 NBUD cases filed at the Children's Oncology Group Neuroblastoma Pathology Ref-erence Laboratory, and survival rates of the patients were analyzed with known prognostic factors. Immunostainings for MYCN and MYC protein were performed on 68 tumors. RESULTS: NBUD cases had a poor prognosis (48.4% ± 5.0% 3-year event-free survival [EFS]; 56.5% ±5.0% overall survival [OS]), and were often associated with high mitosis-karyorrhexis index (MKI, 65%), prominent nucleoli (PN, 83%), >18 months of age (75%), MYCN amplification (.MYCN-A, 83%), diploid pattern (63%), and IpLOH (loss of hetero-zygosity (72%). However, these prognostic indicators, except for MYCN status, had no significant impact on survival. Surprisingly, EFS for patients with MYCN-A tumors (53.4% ± 5.6%) was significantly better (P = .0248) than for patients with MVC/V-nonamplified (.MYCN-NA) tumors (31.7% ± 11.7%), with MYCN-NA and PN ( + ) tumors having the worst prognosis (9.3% ± 8.8%, P = .0045). Immuno-histochemically, MYCN expression was found in 42 of 48 MYCN-A tumors. In contrast, MYC expression was almost exclusively found in the MYCN-NA tumors (9 of 20) especially when they had PN (8 of 11). Those patients with only MYC-positive tumors had the worst EFS (N = 8,12.5% ± 11.7%) compared with only MYCN-positive (N = 39, 49.9% ± 17.7%) and both negative tumors (N = 15, 70.0% ± 17.1%) (P = .0029). High MKI was often found in only MYCN-positive (30 of 38) but rarely in only MYC-positive (2 of 8) tumors. CONCLUSIONS: NBUD represents a unique subtype of neuroblastoma associated with a poor prognosis. In this subtype, MYC protein expression may be a new prognostic factor indicating more aggressive clinical behavior than MYCN amplification and subsequent MYCN protein expression.
- Subjects
NEUROBLASTOMA; NUCLEAR proteins; GENE expression; MYC proteins; CANCER immunology; IMMUNOHISTOCHEMISTRY; PROGNOSIS
- Publication
Cancer (0008543X), 2013, Vol 119, Issue 20, p3718
- ISSN
0008-543X
- Publication type
Article
- DOI
10.1002/cncr.28251